Definition of Schizencephaly

Schizencephaly: A developmental disorder of the brain characterized by abnormal slits, or clefts, in the cerebral hemispheres. Schizencephaly is a form of porencephaly.

Individuals with clefts in both hemispheres, or bilateral clefts, are often developmentally delayed and have delayed speech and language skills and corticospinal dysfunction. Individuals with smaller, unilateral clefts (clefts in one hemisphere) may be weak on one side of the body and may have average or near-average intelligence. Patients with schizencephaly may also have varying degrees of microcephaly, mental retardation, hemiparesis (weakness or paralysis affecting one side of the body), or quadriparesis (weakness or paralysis affecting all four extremities), and may have reduced muscle tone (hypotonia). Most patients have seizures and some may have hydrocephalus.

In schizencephaly, the neurons border the edge of the cleft implying a very early disruption in development. There is now a genetic origin for at least one type of schizencephaly. Other possible causes may include environmental exposures during pregnancy such as medication taken by the mother, exposure to toxins, or a vascular insult. Often there are associated heterotopias (isolated islands of neurons) which indicate a failure of migration of the neurons to their final position in the brain.

Treatment for individuals with schizencephaly generally consists of physical therapy, treatment for seizures, and, in cases that are complicated by hydrocephalus, a shunt.

The prognosis for individuals with schizencephaly varies depending on the size of the clefts and the degree of neurological deficit.


Last Editorial Review: 6/14/2012

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